Molecular weight (DA)
52kD
Immunogen
Synthesized peptide derived from part region of human protein
Specificity
CHST3 Polyclonal Antibody detects endogenous levels of protein.
Formulation
Liquid in PBS containing 50% glycerol, and 0.02% sodium azide.
Dilution rate
WB 1:500-2000 ELISA 1:5000-20000
Purification process (Immunogen)
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Background
This gene encodes an enzyme which catalyzes the sulfation of chondroitin, a proteoglycan found in the extracellular matrix and most cells which is involved in cell migration and differentiation. Mutations in this gene are associated with spondylepiphyseal dysplasia and humerospinal dysostosis. [provided by RefSeq, Mar 2009],
Function
catalytic activity:3'-phosphoadenylyl sulfate + chondroitin = adenosine 3',5'-bisphosphate + chondroitin 6'-sulfate.,disease:Defects in CHST3 are a cause of humerospinal dysostosis (HSD) [MIM:143095]. HSD is characterized by bifurcation of the ends of the humerus, subluxation in the elbow joints, widened iliac bones, talipes equinovarus and coronal cleft vertebrae. Congenital, progressive heart disease, possibly with fatal outcome, is observed in some patients.,disease:Defects in CHST3 are the cause of spondyloepiphyseal dysplasia Omani type (SED Omani type) [MIM:608637]. SED Omani type is an autosomal recessive disorder characterized by normal length at birth but severely reduced adult height (110-130 cm), severe progressive kyphoscoliosis, arthritic changes with joint dislocations, genu valgum, cubitus valgus, mild brachydactyly, camptodactyly, microdontia and normal intelligence. As a
Protein name
Carbohydrate sulfotransferase 3 (EC 2.8.2.17) (Chondroitin 6-O-sulfotransferase 1) (C6ST-1) (Chondroitin 6-sulfotransferase) (Galactose/N-acetylglucosamine/N-acetylglucosamine 6-O-sulfotransferase 0)
Fields
>>Glycosaminoglycan biosynthesis - chondroitin sulfate / dermatan sulfate
Human protein sequence Database
Q7LGC8
Mouse protein sequence database
O88199
Rat protein sequence database
Q9QZL2
Cellular localization
Golgi apparatus membrane ; Single-pass type II membrane protein .
Tissue expression
Widely expressed in adult tissues. Expressed in heart, placenta, skeletal muscle and pancreas. Also expressed in various immune tissues such as spleen, lymph node, thymus and appendix.
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